Evaluation of HbxRBC product as a screening method in sickle cell Anemia
Keywords:
Sickle cell anemia, Hb*RBC, Sickle cell disease
Abstract
Background: India has been ranked the second worst affected country in terms of predicted SCD births. In developed countries, new-born screening programs have been established to ensure early diagnosis, but in most developing countries, screening is not performed, and the diagnosis is often delayed. Aim of present study was to analyse the effectiveness of Hb*RBC product as a screening tool.
Methods: In the present study, blood from 200 patients suspected to have Sickle cell hemoglobinopathies was analysed and subjected it to Sickling screening test. All positive cases were subjected to HPLC to separate constituent haemoglobins and CBC analysis was done to check RBC indices. Hb*RBC product was analysed.
Results: Analysis of Hb*RBC product revealed that there was a statistically significant difference in values between controls and disease patients. Hb*RBC product had good sensitivity and specificity in predicting sickle cell disease.
Conclusions: Hb*RBC<45 was sensitive and specific predictor of sickle cell disease and can be used as a screening tool. Patients with this finding should be further subjected to specific tests like Hb electrophoresis for confirmation.
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References
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22. Pearson HA. A neonatal program for sickle cell anemia. Adv Pediatr. 1986;33:381-400.
23. Scott RB, Harrison DL. Screening of the umbilical cord blood for sickle cell disease: utilization and implementation. Am J Pediatr Hematol Oncol. 1982 Summer;4(2):202-205.
24. Odunvbun ME, Okolo AA, Rahimy CM. Newborn screening for sickle cell disease in a Nigerian hospital. Public Health. 2008;122(10):1111-1116. doi: 10.1016/j.puhe.2008.01.008. Epub 2008 May 19.
25. Walke VA, Walde MS. Haematological study in sickle cell homozygous and heterozygous children in the age group 0-6 years. Indian J Pathol Microbiol. 2007;50(4):901-904.
26. Chikhlikar K, Wilkinson A. A study of red cell parameters in patients of sickle cell trait. IOSR J Dental Medical Sci. 2014;13(3):46-50.
27. Pathak K, Kishore S, Anshu, Shivkumar VB, Gangane N, Sharma S. Study of haemoglobin S percentage and haematological parameters in sickle cell trait. Indian J Pathol Microbiol. 2003;46(3):420-424.
2. Wilson M, Forsyth P, Whiteside J. Haemoglobinopathy and sickle cell disease. Continuing Education in Anaesthesia, Critical Care & Pain. 2009;10(1):24-28. doi:10.1093/bjaceaccp/mkp038.
3. Khan Y, Thakur AS, Mehta R, Kundu RK, Agnihotram G. Hematological profile of sickle cell disease: a hospital-based study at Cims, Bilaspur, Chhattisgarh. IJABPT. 2010;1(2):717-721.
4. Hockham C, Bhatt S, Colah R, Mukherjee MB, Penman BS, Gupta S, et al. The spatial epidemiology of sickle-cell anaemia in India. Sci Rep. 2018;8(1):17685. doi: 10.1038/s41598-018-36077-w.
5. Rao VR. Genetics and epidemiology of sickle cell anemia in India. Indian J Med Sci. 1988;42(9):218-222.
6. Jain DL, Sarathi V, Upadhye D, Gulhane R, Nadkarni AH, Ghosh K, et al. Newborn screening shows a high incidence of sickle cell anemia in Central India. Hemoglobin. 2012;36(4):316-322. doi: 10.3109/03630269.2012.691434. Epub 2012 Jun 19.
7. Rupani MP, Vasava BC, Mallick KH, Gharat VV, Bansal R. Reaching community through school going children for sickle cell disease in Zankhvav village of Surat district, Western India. Online J Health Allied Sci. 2012;11(2).
8. Sickle Cell Anemia Control Project, Ahmedabad. Government of Gujarat 2012. Available at http://www.sickle-cell.gujarat.gov.in/SC_Program.aspx. [Last accessed on 2016 May 28]
9. Jawarkar A, Bhatia V. A study of HPLC patterns in patients of sickle cell anemia with analysis of red cell parameters. Int J Res Med Sci. 2018;6(7):2390-2395. doi: http://dx.doi.org/10.18203/2320-6012.ijrms20182823.
10. Valavi E, Ansari MJ, Zandian K. How to reach rapid diagnosis in sickle cell disease? Iran J Pediatr. 2010;20(1):69-74.
11. Odièvre MH, Verger E, Silva-Pinto AC, Elion J. Pathophysiological insights in sickle cell disease. Indian J Med Res. 2011;134(4):532-537.
12. Lane PA. Sickle cell disease. Pediatr Clin North Am. 1996;43(3):639-664. doi: 10.1016/s0031-3955(05)70426-0.
13. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med. 1997;337(11):762-769. doi: 10.1056/NEJM199709113371107.
14. Bainbridge R, Higgs DR, Maude GH, Serjeant GR. Clinical presentation of homozygous sickle cell disease. J Pediatr. 1985;106(6):881-885. doi: 10.1016/s0022-3476(85)80230-4.
15. Williams S, Maude GH, Serjeant GR. Clinical presentation of sickle cell-hemoglobin C disease. J Pediatr. 1986;109(4):586-589. doi: https://doi.org/10.1016/S0022-3476(86)80217-7.
16. O'Brien RT, McIntosh S, Aspnes GT, Pearson HA. Prospective study of sickle cell anemia in infancy. J Pediatr. 1976;89(2):205-210. doi: 10.1016/s0022-3476(76)80449-0.
17. Leikin SL, Gallagher D, Kinney TR, Sloane D, Klug P, Rida W. Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease. Pediatr. 1989;84(3):500-508.
18. Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle-cell disease in Jamaica. Br Med J (Clin Res Ed). 1982;285(6342):633-635. doi: 10.1136/bmj.285.6342.633.
19. Vichinsky E, Hurst D, Earles A, Kleman K, Lubin B. Newborn screening for sickle cell disease: effect on mortality. Pediatr. 1988;81(6):749-755.
20. Githens JH, Lane PA, McCurdy RS, Houston ML, McKinna JD, Cole DM. Newborn screening for hemoglobinopathies in Colorado. The first 10 years. Am J Dis Child. 1990;144(4):466-470. doi: 10.1001/archpedi.1990.02150280088018.
21. Vichinsky EP. Comprehensive care in sickle cell disease: its impact on morbidity and mortality. Semin Hematol. 1991;28(3):220-226.
22. Pearson HA. A neonatal program for sickle cell anemia. Adv Pediatr. 1986;33:381-400.
23. Scott RB, Harrison DL. Screening of the umbilical cord blood for sickle cell disease: utilization and implementation. Am J Pediatr Hematol Oncol. 1982 Summer;4(2):202-205.
24. Odunvbun ME, Okolo AA, Rahimy CM. Newborn screening for sickle cell disease in a Nigerian hospital. Public Health. 2008;122(10):1111-1116. doi: 10.1016/j.puhe.2008.01.008. Epub 2008 May 19.
25. Walke VA, Walde MS. Haematological study in sickle cell homozygous and heterozygous children in the age group 0-6 years. Indian J Pathol Microbiol. 2007;50(4):901-904.
26. Chikhlikar K, Wilkinson A. A study of red cell parameters in patients of sickle cell trait. IOSR J Dental Medical Sci. 2014;13(3):46-50.
27. Pathak K, Kishore S, Anshu, Shivkumar VB, Gangane N, Sharma S. Study of haemoglobin S percentage and haematological parameters in sickle cell trait. Indian J Pathol Microbiol. 2003;46(3):420-424.
CITATION
DOI: 10.17511/jopm.2019.i11.06
Published: 2019-11-30
How to Cite
Jawarkar, A., & Agrawal, A. (2019). Evaluation of HbxRBC product as a screening method in sickle cell Anemia. Tropical Journal of Pathology and Microbiology, 5(11), 868-871. https://doi.org/10.17511/jopm.2019.i11.06
Section
Original Article
OAI - Open Archives Initiative
